Autoimmune hepatitis and overlap syndrome among Alaska Native people: Prevalence, clinical characteristics, and remission

Abstract

Background and AimHigh autoimmune hepatitis (AIH) and overlap syndrome (OS) prevalence have been previously documented among Alaska Native people. The purpose of this project is to report changes in AIH/OS prevalence over time, clinical characteristics, and factors associated with biochemical remission.MethodsWe reviewed medical records for Alaska Native/American Indian (AN/AI) patients diagnosed with AIH/OS between 1984 and 2021. Point prevalence was calculated based on AIH/OS patients alive at the end of 2021 and at 5‐year intervals from July 1, 2000, to July 1, 2020.ResultsWe identified 189 AN/AI persons diagnosed with AIH or OS (157 AIH, 32 OS). Of these 189, 137 were alive at the end of 2021 for a point prevalence of 91.2 per 100 000 (95% confidence interval [CI]: 77.2–107.8)—75.9 (95% CI: 63.2–91.2) for AIH and 15.3 (95% CI: 10.2–23.0) for OS. Prevalence for both AIH and OS has risen steadily since 2000. Eighty‐nine consented participants (62.7%) achieved biochemical remission with a median time from diagnosis to start of remission of 1.9 years (IQR 0.5–5.0 years). Consented patients with fatty liver were less likely to achieve remission, but their time to remission was shorter than for patients without fatty liver.ConclusionThe AN/AI population in Alaska continues to have the highest reported prevalence of AIH/OS in the world, with prevalence rising steadily since 2000. High reported AIH/OS prevalence is likely due in part to strong referral networks for liver disease. Detection and treatment can lead to biochemical remission and improved health outcomes.

The prevalence and disease course of autoimmune liver diseases in Greenland

“…Regarding AIH, the prevalence of 12.3 per 100,000 is lower than reported for most North European countries, including Denmark with a prevalence of 23.9 per 100,000 [ 12 , 24 ]. Notably, AIH is also less prevalent in Greenland than among Inuit in Alaska, who are included in the “Alaska Natives” minority population with a record-high AIH prevalence of 91.2 per 100,000 [ 11 ]. Hence, although Inuit in Greenland and Alaska share unique genetics, this seems to have little influence on the susceptibility to develop AIH.…”

“…Therefore, the burden of autoimmune diseases may also resemble that of Western countries, as recently reported for autoimmune inflammatory bowel diseases [ 9 ]. Hepatologists practicing in Circumpolar areas refer to studies investigating AIH, PBC, PSC, and AIH with overlap disease in Alaska natives [ 10 , 11 ]. These studies reported a high and rising prevalence of AIH and AIH with overlap disease and a similar prevalence of PBC compared to Scandinavian countries, while no patients with PSC were identified [ 10–14 ].…”